‘Groveroid’ Darier's disease?

Unfortunately, the toxicity of cyclophosphamide, manifested as haemorrhagic cystitis, bladder fibrosis, bone-marrow suppression, ovarian failure and neoplasms, limits its longterm use. In addition, long-term use of azathioprine not infrequently results in potential life-threatening side-effects including severe cytopenia with thiopurine methyltransferase deficiency, hypersensitivity syndrome with liver failure and pancreatitis. Mycophenolate mofetil represents a new immunosuppressive drug which has been shown to be efficacious in the prevention of graft rejection in organ-transplanted patients. However, the use of mycophenolate mofetil has been rapidly expanded for the treatment of autoimmune and inflammatory skin diseases. Mycophenolate mofetil is the morpholinyl ethyl ester prodrug of mycophenolic acid, initially isolated from Penicillium sp. It interferes with de novo purine synthesis by inhibiting type II inosine monophosphate dehydrogenase, an enzyme expressed in stimulated T and B lymphocytes. Mycophenolate mofetil is usually well tolerated. The principal adverse reactions associated with mycophenolate mofetil, including diarrhoea, nausea, vomiting, abdominal pain, reversible mild anaemia, leucopenia and infections, are generally seen in patients receiving more than 2 g daily or who subsequently receive other immunosuppressive drugs. Long-term results over 13 years have shown no increased risk of malignancies in patients treated with mycophenolate mofetil. In our patient the combination therapy resulted in rapid clinical remission and allowed tapering of the prednisone. Furthermore, the asthma, which usually persists in all stages of CSS, showed a significant improvement. Treatment with mycophenolate mofetil was tolerated by our patient without any side-effects. Mycophenolate mofetil may present a promising new agent for mechanism-based treatment of patients with ANCAassociated vasculitis in whom either standard therapy has failed or contraindications for standard therapy have developed.

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